Download - Kuliah Mov Disorder

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MOVEMENT DISORDER


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THE EXTRAPYRAMIDAL SYSTEM

DEFINITION: The extrapyramidal system is a general

term for the basal ganglia

STRUCTURE:

Centers: the corpus striatum, the substantianigra, thalamic and subthalamic nuclei

Inter connections: the above centers areinterconnected with: The cerebral cortex, the

cerebellum, the reticular formation, the cranialnerve nuclei (particularly the vestibular nerve)and the spinal cord


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Functions of the extrapyramidal

system

Regulation of voluntary motor activity

Control of the muscle tone

Maintenance of emotional and associative

movements


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Movement disorders

Hypokinetic

Idiopathic Parkinson's diseaseParkinsonism

Parkinsonism-plus

Hyperkinetic

Essential tremor

Dystonia

Hemiballismus/Ballismus

Chorea-atetosa

Myoclonuus

Hemiballismus

TIC


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PARKINSONISM

Parkinsonism (shaking palsy) is a condition in

which there are static regular tremors,

hypertonia of the muscles of the body withbradykinesia and postural instability

Parkinson Disease : idiophatic Parkinsonism

doe to damage on Subtansia nigra andStriatum


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PARKINSON DISEASE

RESTING TREMOR ( T )

RIGIDITY ( R )

AKINESIA ( A )

POSTURAL INSTABILITY ( P )


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History of Parkinsons disease (PD)

First described in 1817 by an English physician,

James Parkinson, in An Essay on the Shaking

Palsy.

The famous French neurologist, Charcot, further

described the syndrome in the late 1800s.


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Epidemiology of PD

The most common movement disorder

affecting 1-2 % of the general

population over the age of 65 years.

The second most common

neurodegenerative disorder afterAlzheimers disease (AD).


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Risk factors of PD

Age -the most important risk factor

Positive family history

Male gender

Environmental exposure: Herbicide and pesticideexposure, metals (manganese, iron), well water, farming,

rural residence, wood pulp mills; and steel alloy

industries

Race

Life experiences (trauma, emotional stress, personality

traits such as shyness and depressiveness)?

An inverse correlation between cigarette smoking and

caffeine intake in case-control studies.


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Functional neuroanatomy of PD

Substantia nigra: The major origin of the dopaminergic

innervation of the striatum.

Part of extrapyramidal system which processes

information coming from the cortex to the striatum,

returning it back to the cortex through the thalamus.

One major function of the striatum is the regulation of

posture and muscle tonus.


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Dopamine pathways in human brain


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Dopamine synthesis


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PATHOGENESIS

Changes in neuromuscular profile occur as follows:

1.Putamen/Striatum:

Dopamine 60%

Noradrenaline 60%

5- hydroxytryptamine 60%2. Substantia nigra:

Dopamine 60%

Glutamic acid decarboxylate (GDA)+-amino butyric acid

(GABA)3. Cerebral cortex

Glutamic acid decarboxylate (GDA)+-amino butyric acid(GABA)


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Acethylcholine

PARKINSON A CRITICAL BALANCE

IDopamine

Feb 4, 2005


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WHEN TO START TREATMENT FOR

PARKINSON DISEASE

WHEN DISEASE MANIFESTATIONS INTERFERE

WITH SOCIAL AND VOCATIONAL ACTIVITIES,WORSENING OR GAIT OR BALANCE OR OTHER

ACTIVITIES OF DAILY LIVING.

PARTNERSHIP WITH PATIENT!


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TREATMENT OF

PARKINSON DISEASE

MEDICAL

DOPAMINERGIC AGENTS

DOPAMINE AGONIST

ANTI-CHOLINERGICS

SURGICAL

ABLATIVE

Talamotomy.Pallidotomy

D.B.S.(Deep Brain

Stimulation )

PHYSICAL THERAPIES

P.T.

O.T.

SPEECH

EXERCISE Rx, TAI-CHI

PSYCHOTHERAPIES

COUNSELLING

SOCIAL WORK


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Strategy of Treatment

(A) DopamineCarbidopa/l-dopa

Dopamine agonists: Apomorphine,

CabergolineRopinirole,Pramipexole

COMT inhibitors: Entacapone

MAO Inhibitors: Selegiline

Inhibitors of dopamine re-uptake: Amantadine


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Strategy of Treatment

(2) Acetylcholine

Anticholinergic

Antihistaminics


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TREATMENT OF

PARKINSON DISEASE

Drugs

DOPAMINERGIC AGENTS

L-Dopa, L-Dopa+ Benzeraside,

L-Dopa+ Benzeraside+ Entacapone

DOPAMINE AGONIST

Pramipexole, Ropinelrole

ANTI-CHOLINERGICS

Trihexyphenidyl


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WHAT ABOUT LEVODOPA/L-DOPA

STILL THE BEST, ESPECIALLY SHORT TERM

LONG TERM USE . Side effect

MOTOR FLUCTUATIONS, DYSKINESIASON-OFF Phenomenon, WEARING -OFF

FIRST CHOISE

BUT NEARLY ALL PATIENTS EVENTUALLY REQUIRE IT

ANTICHOLENERGIC : Good for Tremor, but NotGOOD, fore Age > 70 years


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Essential Tremor

Bilateral action tremor of the hands andforearms

Absence of other neurologic signs

Long duration (>3 years)

Family history

Beneficial response to ethanol


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Tremor

Rest tremor Action tremor

PD

Tardive dyskinesia

Severe ET

Physiologic

ET

PD

Midbrain

Cerebellar

Handwriting

Postural Kinetic Task-Specific


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Classification Age of onse

Distribution

Etiology


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Classification: Age of Onset Early Onset

childhood, adolescence.

Late Onsetadulthood (peak 30 50 years).


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Classification: Distribution Focal Dystonia affecting a single body part or

location

Segmental Dystonia affecting adjacent body

parts

Hemidystonia affecting one side of the body

Generalized affecting more than 2 segment

of the body


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Classification: Etiology1.Primary dystonia

No structural abnormality in the CNS (oftengenetic)

2.Dystonia Plus Syndromes Primary dystonia + parkinsonism, myoclonus

3.Secondary Dystonia

demonstrable exogenous or structural4.Heredodegenerative Dystonia

underlying brain degeneration


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Treatment

Focal / Segmental

Botulinum toxin injections to affected areas

Medications

Primary Generalized

Oral Medications

Intrathecal baclofen

Surgery


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Medications

Levodopa (Sinemet)

Everyone should be given a trial of sinemet

especially children.

Treatment of choice for Dopa-Responsive Dystonia(low doses 100 to 300 mg)

Other causes of dystonia may respond to

levodopa


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Medications

Anticholinergics

Trihexiphenidyl (Artane).

Start at 1 mg daily and increase weekly to effect,

in children may go up to 30 or 40 mg. Not tolerated well in adults.

Side effects - confusion, drowsiness,hallucinations,forgetfulness, personality changes,

dry mouth, blurred vision, and urinary retention. One poorly designed RTC showed 50 %

improvement.


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Medications

Dopamine Depletors/ Blockers

Tetrabenzine.

Clozapine.

Olanzapine.

Benzodiazepines

Baclofen (oral and intrathecal)


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Surgery

Pallidal Deep Brain Stimulation

Series only ~ 50 % improvement.

Better results with primary generalized dystonia.

Mixed results with secondary generalized

dystonia.

Series only, no RTCs.

Effects delayed by months after surgery.


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Drug-Induced Movement Disorders

Neuroleptic

Parkinsonism

Acute dystonia

Tardive dyskinesia

Neuroleptic Malignant Syndrome

Central Anti emetics

Dystonia


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Non Medication Treatment

Occupational focal dystonia.

Supportive therapy.

Physiotherapists, occupational therapist, speech

therapists.

Education, support groups.


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